Dr. Google, Grisel's Syndrome

[Myra Pumphrey]

y’all – I received this interesting email this week (the name’s have been changed to protect the innocent).

Hey Myra!

Your office was kind enough to find an appt w u so I can bring in Sarah to start an eval on her neck.

She had an adenoidectomy in March and had some post surgical complications – neck pain and infection. She was treated w antibiotics and anti-inflammatories but she still has one symptom we need help with.

She has the urge to crack her neck on the side where the inflammation was and it is loud. This had onset immediately after surgery and was frequent, severe and painful. It has diminished in frequency and she says it isn’t painful just uncomfortable. They think she had Grisel’s syndrome a rare complication from adenoid and apparently other head surgeries like cochlear implants.

Just wanted to send this geeky article on it before I forget. Looking forward to seeing you and so very grateful to be able to get your take on this. Catherine

Have a read of the article.

What do you think of the article?
Have you ever heard of Grisel’s Syndrome before reading this article?

Upon observation, this 10 y.o. patient is in no distress, forward head posture with no rotation or sidebend component. Her complaint is: 1) She is sore and tight in the right upper thoracic region; 2) her neck cracks in the R upper cervical region 2 x/day and there is no pain with the cracking. Decreased frequency and intensity of cracking over the past several weeks. Pt. turns her head to the right to the end of her range to demonstrate the ‘crack’ (65 deg to the R, same as rotation L which is asymptomatic)

What is YOUR level of fear for this patient? Your initial hypothesis?
What would be some specific questions you would want to ask in the subjective exam to address yellow/red flags?

Attachments:

You must be logged in to view attached files.

[Nick Law]

Myra,

Thank you for posting this. I most certainly had not heard of Grisel’s syndrome prior to this post and the associated article.

As an aside, I think it is impressive and encouraging that you would have a mother want to bring their 8 year old child in with a potentially serious condition to get your input.

The child you are seeing seems to have a history and presentation that could certainly be consistent with a “lower grade” Grisel’s syndrome.

I would certainly be asking a whole host of questions and performing objective testing regarding neurologic symptoms, (cranial nerve, myelopathic signs/symptoms, etc…) as well as other upper cervical dysfunction/instability symptoms (e.g., headaches).

Although I would certainly want to get as much of the above information as possible, in light of the area of potential pathology and the risk of potentially serious consequences, I would proceed very cautiously regardless of what objective testing revealed and would advise imaging studies to be performed with potential immobilization for a defined period of time. I would probably advise against all activities that would place the child at risk for further instability progression.

My level of fear – as if this child has poor prognostic outcome – is not too high as from the information presented it seems she will recover well conservatively; however my level of concern and alertness would be very high due to the possibility of progression of symptoms and long-term consequences.

[Kyle Feldman]

Crazy diagnosis.

I do not feel her presentation was anywhere near the level of the patient presented in the case (no torticollis, gross rotational limitations) but until we saw every image I would not rule it out at all.

I agree with Nick about being very conservative even without neuro signs just because it is something we are not familiar with and the risk of death in past cases.

Thank you

[Myra Pumphrey]

Thank you for your thoughts! As it turns out, the patient’s had a ‘knot’ in the right upper cervical region after her surgery. She also had some mild discomfort in the upper cervical region which improved within a week. The Mom asked the ENT about the knot and the ENT brought up the concern about Grisel’s. He ordered a CT which was negative for any asymmetry but they saw something in the R upper cervical region that they thought may be consistent with an infection. She was then treated with antibiotics. She never had torticollis. She had no other complaints w/ clearing questions for headaches, symptoms related to cranial nerve, spinal cord or VBI, except, she did have intermittent ringing in the R ear which was present prior to surgery and was improving gradually since surgery. She said there is no relation of ringing in the ear to head movement. I will continue to monitor this symptom closely. When asked, her Mom said the ENT said this is typical for her symptoms which lead to adenoidectomy. When I asked the Mom if the doctor thought she had Grisel’s Syndrome after the CT, she said ‘Well,…no’.

The patient has been fully active since her initial recovery from the surgery and has had no hesitation to move her neck or play with her friends.

More on the findings with instability testing later…

From what you know so far, do the features fit for instability?

M

[Laura Thornton]

To answer your question on instability features of our patient,

For upper cervical instability features:

– Forward head posture
– Intermittent ringing in the ears
– Minor trauma to neck (surgery)

Denies headaches, dizziness, or other cranial/VBI symptoms.

For segmental instability:
– self-manipulation temporarily relieves symptoms until feels “tight” again, especially with prolonged sitting postures.
– location of pain (R U/T)

A lot of the research for Grisel’s syndrome are case reports of patients within a very acute period after surgery, with minimal information on long-term results or outcomes. Since she is four months out of surgery, how would this change everyone’s view on evaluation and treatment? I agree with you all that I would certainly continue to monitor all signs/symptoms and proceed cautiously, because of this lack of evidence and potential for serious consequences.

[Myra Pumphrey]

Hi all – Today may be my last posting for a while as I am headed into the Canadian Rockies tomorrow!

I agree with all that there should be some level of caution due to the history, but, as pointed out by many of you, the features do not really fit with instability. Also, much of the description by the patient’s Mom is a bit ramped-up compared to the daughter’s description of the symptoms and history. It would be easy to get wrapped up in Mom’s fear, but a fully active child who does not hesitate to move, never hesitated to move did not give me a high level of concern. The only symptom that concerned me was the ringing in the ears. Interesting,….during instability testing for R Alar ligament, the patient stopped me from doing the test by grabbing my arm with her right hand. All other tests were negative. This gave me significant concern, but surprised me based on the rest of her presentation. I questioned the patient about what she felt and why she grabbed my arm. She said she did not want me to touch her ear (she had been through many uncomfortable exams of her ear over recent months and became fearful of exams. One of the first things she asked me when I came into the room was ‘are you going to look in my ears?’). I moved my hand placement and repeated the test and the test was negative. For sure, we will continue to monitor her ringing in the ears and if she does not improve quickly with treatment, we should order additional testing. On day one, I did some gentle STM and joint mobilization, gr. I, progressing to IV- for hypomobility in the right upper cervical region. Upon reassess, her rotation was improved, no pop. Upon talking to the pt’s Mom on the next day, she noted improvement from the first treatment that was lasting into the next day.

If we became more concerned about instability, what other diagnostic tests may be helpful? M

[Nick Law]

The picture certainly looks less grim with the rest of the physical exam presentation you provide, however I still can’t say that I wouldn’t have been fairly cautious and conservative based on the history.

I presume CT was performed in a neutral head position. Flexion/extension X rays can be used for C1/2 saggital instability. I am not sure they ever perform this, however I wonder if end range rotation X rays would be able to show more than a neutrally oriented X ray.

[Michael McMurray]

Just another case of an MD making our jobs harder.

How can you in your right mind make a statement full of misinformation and FEAR to a mom with a child without adequate information. If there was real concern – open mouth motion series films should have been ordered. The concern should have been brought up after the films if there was any real concern.

When you do Google it – you think your child is about to die. No other presentations in this case except some “cracking” post operative.

Great exam Myra – I hope your treatment is not just repeatedly convincing the parents that there child is not at risk for death. Hopefully the child is young enough not understand the ENT’s inappropriate possible “diagnosis” and just move on as a normal kid doing whatever she wants to.

Another example of how powerful every word we say to patients can be interpreted or misinterpreted, creating unnecessary fear and possible disability.

IFOMPT was amazing – Adelaide Australia 2020 – put it your calendar

Cheers

[Author]

Posts